FRI0287 EFFICACY OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS TREATMENTS ACCORDING TO THE TYPE OF MANIFESTATIONS BASED ON ANALYSIS OF 636 PATIENTS
Authors: Matthias Papo, Renato A. Sinico, Vítor Teixeira, Maria-Letizia Urban, Juliane Mahrhold, Sara Monti, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Bernhard Hellmich, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cécile-Audrey Durel, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) represent the treatment cornerstone. So far, EGPA management has been based on conventional immunosuppressants, but GC-dependence remains frequent. Recently, therapies targeting B cells and interleukin-5 have been prescribed, but data on large cohorts are lacking. <h3>Objectives:</h3> This study aimed to describe therapeutic management and efficacy of treatments in EGPA patients. <h3>Methods:</h3> We set up a multicenter European cohort that included 636 EGPA patients. Treatments used, complete remission rates and vasculitis relapse-free survival were recorded. Complete remission was defined as absence of vasculitis relapse and prednisone dose &lt;5 mg/d at last follow-up. Efficacy to treat GC-dependent asthma/ENT signs was defined as the absence of asthma/ENT symptoms and prednisone dose ≤7.5mg/d within the 6 months after initiation. <h3>Results:</h3> For induction, cyclophosphamide (CYC) was the most frequently prescribed immunosuppressant (36.2%), more often in patients with FFS ≥1 (P&lt;0.0001). GCs alone were used in 37.3%, azathioprine (AZA) in 14.4% and methotrexate (MTX) in 6.2%. No difference was found in the 10-years overall survival between patients with FFS=0, FFS=1 and FFS≥2. Complete remission rates were similar between conventional immunosuppressants (CYC, AZA or MTX) and GCs alone. Vasculitis relapse-free survival was also similar between CYC, AZA or MTX and GCs alone. Similar results were observed for first vasculitis relapse treatments. During follow-up, GC-dependent asthma and/or ENT manifestations were treated with AZA (40%), MTX (25%), mycophenolate mofetil (16%), rituximab (RTX) (21%), CYC (19%), cyclosporine (6%), omalizumab (5.9%) and mepolizumab (5.5%), allowing GC-tapering ≤7.5mg/in 23%, 31%, 17%, 43%, 5%, 71%, 25% and 50%, respectively. Conventional immunosuppressants were mostly used in first and second line, while eosinophil-targeted biotherapies were used in 4<sup>th</sup> or 5<sup>th</sup> lines. <h3>Conclusion:</h3> In EGPA patients, the response to conventional immunosuppressants, in addition to GCs, is often disappointing compared to GCs alone, without clear benefit on complete remission rates and relapse-free survival. In contrast, notwithstanding a small number of treated patients, eosinophil-targeted therapies seemed promising to treat asthma and/or ENT manifestations. <h3>Disclosure of Interests:</h3> Matthias Papo: None declared, Renato A. Sinico: None declared, Vítor Teixeira: None declared, Maria-Letizia Urban: None declared, Juliane Mahrhold: None declared, Sara Monti: None declared, Giulia Cassone: None declared, Franco Schiavon: None declared, Benjamin Seeliger: None declared, Thomas Neumann: None declared, Claus Kroegel: None declared, Matthieu Groh: None declared, Chiara Marvisi: None declared, Maxime Samson: None declared, Thomas Barba: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Bernhard Hellmich Consultant for: Roche, Speakers bureau: Abbvie, MSD, Roche, Novartis, Pfizer, Carlomaurizio Montecucco Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Sanofi, Genzyme, Lilly, MSD, Pfizer, UCB, Carlo Salvarani Grant/research support from: Roche, Consultant for: Eli Lilly and Company, Roche, Abbvie, Jean-Emmanuel Kahn: None declared, Bernard Bonnotte: None declared, Cécile-Audrey Durel: None declared, Xavier Puéchal: None declared, Luc Mouthon: None declared, Loïc Guillevin: None declared, Giacomo Emmi: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared